Background
The thousand mysteries around us would not trouble but interest us, if only we had cheerful, healthy hearts.
–Nietzche
If we all had healthy hearts, the mysteries of the heart would not trouble us; however, constrictive pericarditis certainly has been a mystery and remains a diagnostic challenge to this day.
The history of constrictive pericarditis is replete with some of the most famous names in medicine. Richard Lower described a patient with dyspnea and an intermittent pulse in 1669. Lancisi first reported on the constrictive syndrome in 1828. Corrigan described the pericardial knock in 1842. Kussmaul described his sign and the associated paradoxical pulse in 1873.[1, 2, 3]
Constrictive pericarditis has symptoms that overlap a variety of diseases as diverse as myocardial infarction, aortic dissection, pneumonia, influenza, and connective tissue disorders. This overlap can confuse the most skilled diagnostician. An increased suspicion for constriction helps move it to the top of the broad differential diagnosis and provides for a correct diagnosis and timely therapy.
Constrictive pericarditis occurs when a thickened fibrotic pericardium, of whatever cause, impedes normal diastolic filling. This usually involves the parietal pericardium, although it can involve the visceral pericardium (see Pericarditis, Constrictive-Effusive). Acute and subacute forms of pericarditis (which may or may not be symptomatic) may deposit fibrin, which may, in turn, evoke a pericardial effusion. This often leads to pericardial organization, chronic fibrotic scarring, calcification, and restricted cardiac filling.[4]
The classic diagnostic conundrum of constrictive pericarditis is the difficulty in distinguishing it from restrictive cardiomyopathy (see Cardiomyopathy, Restrictive) and other syndromes associated with elevated right-sided pressures that all share similar symptoms, physical findings, and hemodynamics. Although obtaining a careful history and performing a physical examination remain the cornerstones of evaluation, technologic advances have facilitated diagnosis, particularly with the appropriate use of Doppler echocardiography, high-resolution computed tomography (CT), magnetic resonance imaging (MRI), and invasive hemodynamic measurement.
Pathophysiology
The normal pericardium is composed of 2 layers: the tough fibrous parietal pericardium and the smooth visceral pericardium. Usually, approximately 50 mL of fluid (plasma ultrafiltrate) is present in the intrapericardial space to minimize friction during cardiac motion.[5]
Acute and subacute forms of pericarditis (which may or may not be symptomatic) may deposit fibrin, which may, in turn, evoke a pericardial effusion. This often leads to pericardial organization, chronic fibrotic scarring, and calcification, most often involving the parietal pericardium (see Pericarditis, Constrictive-Effusive).[6]
This thickened fibrotic pericardium, regardless of cause, impedes normal late diastolic filling, distinguishing constrictive from restrictive pericarditis. Since the myocardium is unaffected, early ventricular filling during the first third of diastole is unimpeded, but afterwards, the stiff pericardium affects flow and hemodynamics. In other words, the ventricular pressure decreases rapidly early (producing a steep y descent on right atrial pressure waveform tracings) and then increases abruptly to a level that is sustained until systole ("dip-and-plateau waveform" or "square root sign" seen on right or left ventricular pressure waveform tracings).[7]
The clinical symptoms and classic hemodynamic findings can be explained by early rapid diastolic filling and elevation and equalization of the diastolic pressures in all of the cardiac chambers restricting late diastolic filling, leading to venous engorgement and decreased cardiac output, all secondary to a confining pericardium.
Epidemiology
Frequency
United States
Similar to many diseases that in the past were predominantly infectious in origin, the clinical spectrum of constrictive pericarditis has changed in recent years. Approximately 9% of patients with acute pericarditis for any reason go on to develop constrictive physiology.[8] T he true frequency is therefore dependent on the incidence of the specific causes of pericarditis, but since acute pericarditis is only clinically diagnosed in 1 in 1,000 hospital admissions, the frequency of a diagnosis of constrictive pericarditis is less than 1 in 10,000 hospital admissions.
International
In the developing world, infectious etiologies remain more prominent (tuberculosis has the highest total incidence).
Mortality/Morbidity
- Scant data exist because the disease is rare.
- The underlying disease usually determines the prognosis. Poorer prognoses are associated with malignancy and New York Heart Association (NYHA) class III or IV heart failure symptoms.
- Long-term survival after pericardiectomy depends on the underlying cause. Of common causes, idiopathic constrictive pericarditis has the best prognosis (88% survival at 7 years), followed by constriction due to cardiac surgery (66% at 7 years). The worst postpericardiectomy prognosis occurs in postradiation constrictive pericarditis (27% survival at 7 years). This likely represents confounding comorbidities. Predictors of poor outcomes in patients who undergo pericardiectomy including history of prior radiation, worsening renal function, pulmonary hypertension, systolic heart failure, hyponatremia, and advanced age.[9, 10]
Race
- No race predilection exists for this disorder.
Sex
- Most likely a male predominance exists, with a male-to-female ratio of 3:1 in some studies.
Age
- Cases have been reported in persons aged 8-70 years. Predilection is likely reflective of the underlying disease.
- Historical studies suggest a median age of 45 years, while more recent studies suggest a median age of 61 years. This likely reflects a demographic change that is likely to continue.
Chevers N. Observations on diseases of the orifice and valves of the aorta. Guys Hosp Rep. 1842;7:387-92.
Connolly DC, Mann RJ. Dominic J. Corrigan (1802-1880) and his description of the pericardial knock. Mayo Clin Proc. Dec 1980;55(12):771-3. [Medline].
Fowler NO. Constrictive pericarditis: its history and current status. Clin Cardiol. Jun 1995;18(6):341-50. [Medline].
Brockington GM, Zebede J, Pandian NG. Constrictive pericarditis. Cardiol Clin. Nov 1990;8(4):645-61. [Medline].
Shabetai R. Constrictive pericarditis. In: Shabetai R, ed. The Pericardium. New York, NY: Grune & Stratton; 1981.
Hancock EW. On the elastic and rigid forms of constrictive pericarditis. Am Heart J. Dec 1980;100(6 Pt 1):917-23. [Medline].
Shabetai R. Pericardial Disease: etiology, pathophysiology, clinical recognition, and treatment. New York NY: Churchill Livingstone; 1995:1024-35.
Griffin BP, Topol EJ. Pericardial Disease. In: Manual of Cardiovascular Medicine. 2nd ed. Philadelphia, Pennsylvania: Lippincott Williams and Wilkins; 2004:372-396.
Bertog SC, Thambidorai SK, Parakh K, et al. Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy. J Am Coll Cardiol. Apr 21 2004;43(8):1445-52. [Medline].
Kleynberg RL, Kleynberg VM, Kleynberg LM, Farahmandian D. Chronic constrictive pericarditis in association with end-stage renal disease. Int J Nephrol. 2011;2011:469602. [Medline]. [Full Text].
Babuin L, Alegria JR, Oh JK, Nishimura RA, Jaffe AS. Brain natriuretic peptide levels in constrictive pericarditis and restrictive cardiomyopathy. J Am Coll Cardiol. Apr 4 2006;47(7):1489-91. [Medline].
Leya FS, Arab D, Joyal D, et al. The efficacy of brain natriuretic peptide levels in differentiating constrictive pericarditis from restrictive cardiomyopathy. J Am Coll Cardiol. Jun 7 2005;45(11):1900-2. [Medline].
Yazdani K, Maraj S, Amanullah AM. Differentiating constrictive pericarditis from restrictive cardiomyopathy. Rev Cardiovasc Med. 2005;6(2):61-71. [Medline].
Sengupta PP, Mohan JC, Mehta V, Arora R, Khandheria BK, Pandian NG. Doppler tissue imaging improves assessment of abnormal interventricular septal and posterior wall motion in constrictive pericarditis. J Am Soc Echocardiogr. Mar 2005;18(3):226-30. [Medline].
Appleton CP, Hatle LK, Popp RL. Cardiac tamponade and pericardial effusion: respiratory variation in transvalvular flow velocities studied by Doppler echocardiography. J Am Coll Cardiol. May 1988;11(5):1020-30. [Medline].
Hurrell DG, Nishimura RA, Higano ST, et al. Value of dynamic respiratory changes in left and right ventricular pressures for the diagnosis of constrictive pericarditis. Circulation. Jun 1 1996;93(11):2007-13. [Medline].
Sohn DW, Kim YJ, Kim HS, et al. Unique features of early diastolic mitral annulus velocity in constrictive pericarditis. J Am Soc Echocardiogr. Mar 2004;17(3):222-6. [Medline].
Sengupta PP, Krishnamoorthy VK, Abhayaratna WP, Korinek J, Belohlavek M, Sundt TM, et al. Disparate Patterns of Left Ventricular Mechanics Differentiate Constrictive Pericarditis from Restrictive Cardiomyopathy. J Am Coll Cardiol Img. 2008;1:29-38.
Oh JK, Hatle LK, Seward JB, et al. Diagnostic role of Doppler echocardiography in constrictive pericarditis. J Am Coll Cardiol. Jan 1994;23(1):154-62. [Medline].
Zurick AO, Bolen MA, Kwon DH, Tan CD, Popovic ZB, Rajeswaran J, et al. Pericardial Delayed Hyperenhancement With CMR Imaging in Patients With Constrictive Pericarditis Undergoing Surgical Pericardiectomy A Case Series With Histopathological Correlation. JACC Cardiovasc Imaging. Nov 2011;4(11):1180-91. [Medline].
Amal L, Nawal D, Abdellah Z, Anis S, Fouad Amal W, Abdellatif B, et al. Use of magnetic resonance imaging in assessment of constrictive pericarditis: a Moroccan center experience. Int Arch Med. Oct 19 2011;4(1):36. [Medline].
Hatle LK, Appleton CP, Popp RL. Differentiation of constrictive pericarditis and restrictive cardiomyopathy by Doppler echocardiography. Circulation. Feb 1989;79(2):357-70. [Medline].
Talreja DR, Nishimura RA, Oh JK, Holmes DR. Constrictive pericarditis in the modern era: novel criteria for diagnosis in the cardiac catheterization laboratory. J Am Coll Cardiol. Jan 22 2008;51(3):315-9. [Medline].
Talreja DR, Edwards WD, Danielson GK, et al. Constrictive pericarditis in 26 patients with histologically normal pericardial thickness. Circulation. Oct 14 2003;108(15):1852-7. [Medline].
Imazio M, Antonio B, Roberto C, Ferrua S, Belli R, Maestroni S, et al. Colchicine treatment for recurrent pericarditis (CORP): a randomized trial. Ann Intern Med. Oct 4 2011;155(7):I28. [Medline].
Tuna IC, Danielson GK. Surgical management of pericardial diseases. Cardiol Clin. Nov 1990;8(4):683-96. [Medline].
Ling LH, Oh JK, Schaff HV, et al. Constrictive pericarditis in the modern era: evolving clinical spectrum and impact on outcome after pericardiectomy. Circulation. Sep 28 1999;100(13):1380-6. [Medline].
Maisch B, Seferovic PM, Ristic AD, Erbel R, Rienmuller R, Adler Y, et al. Guidelines on the diagnosis and management of pericardial diseases. European Society of Cardiology. 2004.
Clare GC, Troughton RW. Management of constrictive pericarditis in the 21st century. Curr Treat Options Cardiovasc Med. Dec 2007;9(6):436-42. [Medline].

